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Childhood adrenal gland pheochromocytoma

MedGen UID:
859271
•Concept ID:
C3899677
•
Neoplastic Process
Synonyms: Childhood Adrenal Gland Pheochromocytoma; childhood adrenal gland pheochromocytoma; Childhood Pheochromocytoma; malignant childhood adrenal gland pheochromocytoma; Pediatric Adrenal Gland Pheochromocytoma; Pediatric Pheochromocytoma; pheochromocytoma, childhood
 
Monarch Initiative: MONDO:0044767

Definition

A rare pheochromocytoma of the adrenal gland that occurs during childhood. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChildhood adrenal gland pheochromocytoma

Professional guidelines

PubMed

International consensus statement on the diagnosis and management of phaeochromocytoma and paraganglioma in children and adolescents.
Casey RT, Hendriks E, Deal C, Waguespack SG, Wiegering V, Redlich A, Akker S, Prasad R, Fassnacht M, Clifton-Bligh R, Amar L, Bornstein S, Canu L, Charmandari E, Chrisoulidou A, Freixes MC, de Krijger R, de Sanctis L, Fojo A, Ghia AJ, Huebner A, Kosmoliaptsis V, Kuhlen M, Raffaelli M, Lussey-Lepoutre C, Marks SD, Nilubol N, Parasiliti-Caprino M, Timmers HHJLM, Zietlow AL, Robledo M, Gimenez-Roqueplo AP, Grossman AB, Taïeb D, Maher ER, Lenders JWM, Eisenhofer G, Jimenez C, Pacak K, Pamporaki C
Nat Rev Endocrinol 2024 Dec;20(12):729-748. Epub 2024 Aug 15 doi: 10.1038/s41574-024-01024-5. PMID: 39147856
Extra-adrenal pheochromocytoma: diagnosis and management.
Disick GI, Palese MA
Curr Urol Rep 2007 Jan;8(1):83-8. doi: 10.1007/s11934-007-0025-5. PMID: 17239321
Clinical rounds from the Massachusetts General Hospital. Childhood hypertension: etiology, investigation and treatment.
Clin Pediatr (Phila) 1966 Jan;5(1):39-44. PMID: 5900379

Recent clinical studies

Etiology

Endocrine morbidity in neurofibromatosis 1: a nationwide, register-based cohort study.
Kenborg L, Ebbehoj A, Ejerskov C, Handrup MM, Østergaard JR, Hove H, Doser K, Krøyer A, Mulvihill JJ, Winther JF, Stochholm K
Eur J Endocrinol 2023 Aug 2;189(2):190-198. doi: 10.1093/ejendo/lvad101. PMID: 37542520
Cancer Stem Cells in Pheochromocytoma and Paraganglioma.
Scriba LD, Bornstein SR, Santambrogio A, Mueller G, Huebner A, Hauer J, Schedl A, Wielockx B, Eisenhofer G, Andoniadou CL, Steenblock C
Front Endocrinol (Lausanne) 2020;11:79. Epub 2020 Feb 25 doi: 10.3389/fendo.2020.00079. PMID: 32158431Free PMC Article
Multiple Endocrine Neoplasia Type 2B Presents Early in Childhood but Often Is Undiagnosed for Years.
Makri A, Akshintala S, Derse-Anthony C, Widemann B, Stratakis CA, Glod J, Lodish M
J Pediatr 2018 Dec;203:447-449. Epub 2018 Oct 9 doi: 10.1016/j.jpeds.2018.08.022. PMID: 30314660Free PMC Article
Von Hippel-Lindau and Hereditary Pheochromocytoma/Paraganglioma Syndromes: Clinical Features, Genetics, and Surveillance Recommendations in Childhood.
Rednam SP, Erez A, Druker H, Janeway KA, Kamihara J, Kohlmann WK, Nathanson KL, States LJ, Tomlinson GE, Villani A, Voss SD, Schiffman JD, Wasserman JD
Clin Cancer Res 2017 Jun 15;23(12):e68-e75. doi: 10.1158/1078-0432.CCR-17-0547. PMID: 28620007
Extra-adrenal pheochromocytoma: diagnosis and management.
Disick GI, Palese MA
Curr Urol Rep 2007 Jan;8(1):83-8. doi: 10.1007/s11934-007-0025-5. PMID: 17239321

Diagnosis

International consensus statement on the diagnosis and management of phaeochromocytoma and paraganglioma in children and adolescents.
Casey RT, Hendriks E, Deal C, Waguespack SG, Wiegering V, Redlich A, Akker S, Prasad R, Fassnacht M, Clifton-Bligh R, Amar L, Bornstein S, Canu L, Charmandari E, Chrisoulidou A, Freixes MC, de Krijger R, de Sanctis L, Fojo A, Ghia AJ, Huebner A, Kosmoliaptsis V, Kuhlen M, Raffaelli M, Lussey-Lepoutre C, Marks SD, Nilubol N, Parasiliti-Caprino M, Timmers HHJLM, Zietlow AL, Robledo M, Gimenez-Roqueplo AP, Grossman AB, Taïeb D, Maher ER, Lenders JWM, Eisenhofer G, Jimenez C, Pacak K, Pamporaki C
Nat Rev Endocrinol 2024 Dec;20(12):729-748. Epub 2024 Aug 15 doi: 10.1038/s41574-024-01024-5. PMID: 39147856
Biochemical Diagnosis of Catecholamine-Producing Tumors of Childhood: Neuroblastoma, Pheochromocytoma and Paraganglioma.
Eisenhofer G, Peitzsch M, Bechmann N, Huebner A
Front Endocrinol (Lausanne) 2022;13:901760. Epub 2022 Jul 26 doi: 10.3389/fendo.2022.901760. PMID: 35957826Free PMC Article
Adrenal Tumors in Childhood.
Fuqua JS
Adv Pediatr 2021 Aug;68:227-244. doi: 10.1016/j.yapd.2021.05.015. PMID: 34243855
Multiple Endocrine Neoplasia Type 2B Presents Early in Childhood but Often Is Undiagnosed for Years.
Makri A, Akshintala S, Derse-Anthony C, Widemann B, Stratakis CA, Glod J, Lodish M
J Pediatr 2018 Dec;203:447-449. Epub 2018 Oct 9 doi: 10.1016/j.jpeds.2018.08.022. PMID: 30314660Free PMC Article
Sonographic diagnosis of pheochromocytoma in childhood and adolescence.
Gerdemann C, Deeg KH
Ultraschall Med 2013 Oct;34(5):413-6. doi: 10.1055/s-0033-1346763. PMID: 24278972

Therapy

Endocrine morbidity in neurofibromatosis 1: a nationwide, register-based cohort study.
Kenborg L, Ebbehoj A, Ejerskov C, Handrup MM, Østergaard JR, Hove H, Doser K, Krøyer A, Mulvihill JJ, Winther JF, Stochholm K
Eur J Endocrinol 2023 Aug 2;189(2):190-198. doi: 10.1093/ejendo/lvad101. PMID: 37542520
Childhood sporadic pheochromocytoma: clinical profile and outcome in 19 patients.
Bhansali A, Rajput R, Behra A, Rao KL, Khandelwal N, Radotra BD
J Pediatr Endocrinol Metab 2006 May;19(5):749-56. doi: 10.1515/jpem.2006.19.5.749. PMID: 16789642
The intraoperative medical management of childhood pheochromocytoma.
Ellis D, Gartner JC
J Pediatr Surg 1980 Oct;15(5):655-9. doi: 10.1016/s0022-3468(80)80519-7. PMID: 6108356
Alpha-adrenergic blocking agent in childhood pheochromocytoma.
Gupta S, Ternberg JL, Weldon VV, Pagliara AS
Surgery 1973 Oct;74(4):545-50. PMID: 4729218
Clinical rounds from the Massachusetts General Hospital. Childhood hypertension: etiology, investigation and treatment.
Clin Pediatr (Phila) 1966 Jan;5(1):39-44. PMID: 5900379

Prognosis

Renal and adrenal tumours in children.
McHugh K
Cancer Imaging 2007 Mar 5;7(1):41-51. doi: 10.1102/1470-7330.2007.0007. PMID: 17339140Free PMC Article
Extra-adrenal pheochromocytoma: diagnosis and management.
Disick GI, Palese MA
Curr Urol Rep 2007 Jan;8(1):83-8. doi: 10.1007/s11934-007-0025-5. PMID: 17239321
Malignant adrenal tumors.
Rescorla FJ
Semin Pediatr Surg 2006 Feb;15(1):48-56. doi: 10.1053/j.sempedsurg.2005.11.008. PMID: 16458846
Pheochromocytoma.
Ram CV
Cardiol Clin 1988 Nov;6(4):517-35. PMID: 3067844
The intraoperative medical management of childhood pheochromocytoma.
Ellis D, Gartner JC
J Pediatr Surg 1980 Oct;15(5):655-9. doi: 10.1016/s0022-3468(80)80519-7. PMID: 6108356

Clinical prediction guides

Endocrine morbidity in neurofibromatosis 1: a nationwide, register-based cohort study.
Kenborg L, Ebbehoj A, Ejerskov C, Handrup MM, Østergaard JR, Hove H, Doser K, Krøyer A, Mulvihill JJ, Winther JF, Stochholm K
Eur J Endocrinol 2023 Aug 2;189(2):190-198. doi: 10.1093/ejendo/lvad101. PMID: 37542520
Bilateral pheochromocytoma revealed by acute abdominal pain in a child. A case report.
Raoudha K, Amel T, Nesrine J, Najla S, Samia B, Sana M, Jihene B, Abdellatif N, Samia T, Lamia B
Pediatr Endocrinol Diabetes Metab 2021;27(2):141-145. doi: 10.5114/pedm.2020.101804. PMID: 33853288Free PMC Article
VHL-Related Neuroendocrine Neoplasms And Beyond: An Israeli Specialized Center Real-Life Report.
Szalat A, Oleinikov K, Nahmias A, Meiner V, Ben-Haim S, Atlan K, Lev-Cohain N, Appelbaum L, Gomori M, Mazeh H, Khalaileh A, Pe'er J, Lossos A, Shoshan Y, Grozinsky-Glasberg S, Gross DJ
Endocr Pract 2020 Oct;26(10):1131-1142. doi: 10.4158/EP-2020-0220. PMID: 33471715
Metastatic pheochromocytoma/paraganglioma related to primary tumor development in childhood or adolescence: significant link to SDHB mutations.
King KS, Prodanov T, Kantorovich V, Fojo T, Hewitt JK, Zacharin M, Wesley R, Lodish M, Raygada M, Gimenez-Roqueplo AP, McCormack S, Eisenhofer G, Milosevic D, Kebebew E, Stratakis CA, Pacak K
J Clin Oncol 2011 Nov 1;29(31):4137-42. Epub 2011 Oct 3 doi: 10.1200/JCO.2011.34.6353. PMID: 21969497Free PMC Article
Pheochromocytoma in children.
Ciftci AO, Tanyel FC, Senocak ME, Büyükpamukçu N
J Pediatr Surg 2001 Mar;36(3):447-52. doi: 10.1053/jpsu.2001.21612. PMID: 11226993

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